Cerebral Sinovenous Thrombosis (CSVT) is a serious complication of L-asparaginase chemotherapy for leukemia in children. Clinical features of headache, altered consciousness, focal neurological deficit, and seizures developing during or immediately after treatment with L-asparaginase should alert the treating physician to the possibility of CSVT. Immediate imaging of the brain should be done using CT and MRI and the veins should be visualized noninvasively by CT and MR venography. We report two children on induction therapy for acute leukemia who presented with seizures, headache, and altered consciousness. Venous infarcts with and without hemorrhage were seen on CT in one patient and the empty delta sign was seen after contrast injection; however, the early changes were missed by CT. MRI detected dural sinus thrombosis relatively earlier in another patient, while the CT findings were equivocal; in this patient, contrast-enhanced MRI showed the empty delta sign and MR venography confirmed absent flow in the superior sagittal sinus, which was diagnostic of sinus thrombosis. Rapid anticoagulation was started with heparin and maintained with warfarin. The child with a unilateral small nonhemorrhagic infarct made a complete recovery while the other, with bilateral hemorrhagic infarcts, did not survive. We stress the importance of early diagnosis of CSVT using CT and MRI in children with leukemia being treated with L-asparaginase; this will permit timely treatment.

Background : The pattern of pediatric stroke displays ethnic and geographical variations. There are few reports from black Sub-Saharan Africa, although relevant data are important in prevention, clinical diagnosis, treatment and prognostication. Aim : To describe subtypes, risk factors, localization, age and gender distribution of pediatric stroke in the black Kenyan population. Study Design and Setting : Retrospective cross-sectional study in a single regional referral and teaching hospital. Statistical Analysis : Data were analyzed by SPSS version 13.0 for Windows and presented in tables and bar and pie charts. Materials and Methods : The study was performed at the Kenyatta National Hospital, a level-6 regional referral health facility with an annual pediatric in-patient turnover of about 40,000 patients. Files of patients aged 1 month to 18 years over a period of 5 years were analyzed for stroke subtypes, localization, risk factors, age and sex distribution. Only those files with complete information were included. Results : Thirty-two of the 712 stroke patients (4.5%) were pediatric. The male:female ratio was 1.7:1. Ischemic stroke comprised 56.3% (n = 18). Mean age was 7.7 years (range, 1.5-18 years). The most common sites were cortical (51%), lacunar (41%) and brain stem (8%). The most common risk factors were connective tissue disorders (28.1%), heart disease (25%), human immunodeficiency virus (9.4%) and infection (9.4%). Conclusion : Pediatric stroke is not uncommon in the Kenyan population. The risk factor profile comprising connective tissue disorders and infection differs from that reported in other populations, inviting large community-based studies.

It is presently a universal practice to administer vitamin K at birth. Hence, the serious bleeding manifestations from vitamin K deficiency are nowadays very rare. We describe a case of late vitamin K deficiency bleeding presenting as intracranial hemorrhage with impending coning and the related review of literature. Such severe bleeding episodes due to vitamin K deficiency are associated with multiple cranial involvement and impending brain herniation is probably rare.

Background: In Acute Myeloid Leukemia (AML), malignant clones of immature myeloid cells (primarily blasts) proliferate, replace bone marrow, circulate in blood and invade other tissues. The unique presentation of bilateral proptosis and bilateral temporal swelling in AML is being reported. Case Report: A 6-year-old girl presented with low-grade fever, progressively increasing bitemporal swelling and bilateral proptosis. Contrast Enhanced Computed Tomographic (CECT) images revealed enhancing infiltrates occupying the lateral orbital wall, causing proptosis. The infiltrate extended toward the bilateral temporal fossae beneath the temporalis muscle and extradurally beneath the frontal and temporal bones. A high total leucocytic count with immature and deformed cells and, Fine Needle Aspiration Cytology (FNAC) from the temporal swelling, the bone marrow aspirate and biopsy showing leukemic blast cells confirmed the diagnosis of AML. Chemotherapy brought about remission of the disease. Conclusions: To the best of the authors' knowledge, simultaneous presence of both bilateral proptosis and bitemporal swellings have not been previously reported in AML. A peripheral blood smear with bone marrow aspirate and biopsy help in the early detection of AML. Institution of early intervention in this potentially fatal disease is often associated with gratifying survival rates.

The common cause of mental impairment and the wide range of physical abnormalities is balanced chromosome rearrangement. As such, it is difficult to interpret, posing as a diagnostic challenge in human development. We present a unique familial case report with the paternally inherited autosomal-balanced reciprocal translocation involving chromosomal regions 8q and 18q. The etiology of the translocation, i.e. 46,XX,t(8;18)(q22.1;q22) was detected by conventional high-resolution Giemsa-Trypsin-Giemsa-banding and fluorescence in situ hybridization techniques. The father was found to be the carrier of the chromosome defect and also the same was observed in the first female child referred with a history of delayed milestone development. However, the second female child showed normal 46, XX karyotype. This is the first report of reciprocal translocation involving 8q and 18q associated with the delayed milestone development. The reason likely may be due to the rearrangement of genetic material at these breakpoints having a crucial relationship and thus manifesting developmental delay in the progeny. Accordingly, this paper also shows genetic counseling discussion for the cause.

We present a rare case of right parietal sinus pericranii in a 2-year-old female child who presented with a compressible swelling on the right side of the scalp since 3 months of age, with a large head. Magnetic resonance imaging along with venography and conventional angiogram was performed, which confirmed the diagnosis.

Objective : To compare the efficacy and side effects of 3% hypertonic saline and mannitol in the management of raised intracranial pressure in children. Design : Prospective randomized study. Setting : Pediatric intensive care unit (PICU) in a tertiary care hospital. Subject : 200 patients with raised intracranial pressure. Materials and Methods : Patients were randomized into two statistically comparable groups; Group A (n = 98) was treated with mannitol while Group B (n = 100) was treated with 3% hypertonic saline. Group C (n = 2) included those members of Group A in whom serum osmolality ≥320 mosmol/kg and were then treated with 3% hypertonic saline. Both Drugs were given at a loading dose of 5 ml/kg stat followed by 2 ml/kg in every 6 h(both have same osmolarity) for two days in their respective groups. Besides monitoring, blood pressure (NIBP), mean arterial pressure (pre and post 30 min of drug), serum sodium, chloride and osmolality were measured. Intracranial pressure was assessed indirectly by measuring mean arterial ressure "MAP". Student paired 't' test was applied.
Results : Decrease in MAP was highly significant (P<0.001) at 0 h in males 0,6 h in females, and moderately significant at 12,36 h in females and significant(P<0.05) at 6,24,42 h in males of Group B. Decrease in coma hours was a highly significant finding (P<0.001) in Group B. In Group B, serum sodium and chloride increased significantly but remained within acceptable limits. There was no difference in osmolality and mortality (fisher Z). Conclusion : Mannitol has several side effects, 3% hypertonic saline is a safe and effective alternative in managing cerebral edema.

Elephantiasis neuromatosa is the most impressive manifestation of neurofibromatosis type-1 (NF-1). We report a case of NF-1 who presented with elephantiasis neuromatosa of his right leg. Cross-sectional imaging not only assists in the correct diagnosis but also aids in imaging the vasculature of a plexiform neurofibroma, which is essential for proper surgical planning.

Mutations in different types of fibroblastic growth factor receptors (FGFRs) have been associated with a variety of phenotype abnormalities, the common ones being Apert, Crouzon and Pfeiffer syndromes. In this study, we present two representative cases having the Apert and Pfeiffer syndromes, respectively, and discuss their clinical presentation, sequel and surgical implications.

Context : Cerebral palsy (CP) is a heterogeneous group of permanent, non-progressive motor disorders of movement and posture caused by chronic brain injuries. It is the most common cause of physical disability in childhood; spastic cerebral palsy being the most prevalent of its various forms. There is scanty information about the neurophysiologic investigations in children diagnosed as having spastic CP. Aims : The aim of the study was to investigate the relationship between abnormal VEP and BAEP findings with different clinical parameters in children with spastic cerebral palsy. Materials and Methods : Fifteen children with spastic CP in the age range 4 months to 10 years participated in this study. Evaluation of VEPs, brainstem evoked potentials (BAEPs) were performed in all study patients as well as 35 healthy children as controls. The study was conducted after obtaining ethics committee approval and informed consent of parents. Statistical Analysis Used : Significance of difference in the mean values of different parameters in different groups was assessed by Student's "t" test and the P value <0.05 was considered to be significant. All the values were expressed as mean ± 1 Std. Deviation. Results : A significant difference was found in the VEP latencies and amplitude between the subjects with CP and controls. Striking BAEP abnormalities in CP patients include prolongation of absolute latency of wave V, interpeak latencies of III-V and lowered I-V ratio. Abnormal VEPs and BAEPs in children with bilateral spastic cerebral palsy demonstrated a correlation with the presence of moderate to severe developmental delay. Conclusions : The differences in VEPs and BAEPs were determined between CP children and healthy children. The abnormalities found are probably linked to the neurological deficits present in cases of cerebral palsy.

Seizure after laminectomy for spinal procedure is very rare and has not been reported after lipomyelomeningocele surgery beforehand. Here, two cases of seizure following laminectomy for lipomyelomeningocele are reported. The exact etiology of the event is unknown but anesthetic material, pneumocephalus, intracranial hypotension subsequent to cerebrospinal fluid leakage after spinal procedures, spinal-induced seizure and the potential toxic effect of fat molecules could be considered.

Desmoplastic infantile ganglioglioma (DIG) is a rare supratentorial brain tumor occurring mostly before the age of 2 years. It has a good prognosis and total excision of the tumor is curative, necessitating no further treatment. An accurate pathologic diagnosis is crucial. Until now, <60 cases of this tumor type have been reported. Herein, we report a 3-month-old boy with intractable seizure who was found to have DIG after surgery.

Bithalamic gliomas are extremely rare tumors of central nervous system. Although they are usually benign in nature, their outcome is poor because of the involvement of thalamic nuclei and inadequate surgical excision. Surgery is usually done to get tissue for diagnosis. Role of radiotherapy and chemotherapy is questionable. They are unique in their metabolic and neuroradiological properties. We report herein a 6-year-old male of bithalamic astrocytoma (WHO grade 2) who presented with raised intracranial pressure and tremors in right upper limb. The child had a very huge bithalamic mass which was debulked through the interhemispheric transcallosal approach in order to reduce the mass effect. He had a stormy post-operative course to recover gradually.

Intramedullary spinal epidermoid cysts are rare, with only few cases having been reported in the literature. We are reporting a case of a 10-year-old female child who presented with symptoms of meningitis with progressive paraparesis. Magnetic resonance imaging of the spine revealed an intramedullary epidermoid cyst from C6 to D5. Near-total excision of the tumor was performed. Histopathological report confirmed the diagnosis of epidermoid cyst. The patient showed progressive recovery.

Congenital deformities involving the coverings of the nervous system are called neural tube defects (NTDs). NTD can be classified as neurulation defects, which occur by stage 12, and postneurulation defects. Cervical meningocele and myelomeningocele are rare spinal dysraphic lesions. Unlike lumbosacral dysraphic lesions, there is often no neurologic deficits and thus the subtle features of cervical cord tethering may be overlooked on imaging. The presence of meningomyelocele and/or encephaloceles at multiple (two or more) sites along the vertebral axis is a very rare event occurring in <1% of cases. Less than 10 cases have been described in the published literature. We are reporting a case of multiple NTD in same patient with no neurological deficit.

The authors report a pediatric patient who presented with a slow-growing swelling on the scalp. Computed tomography (CT) of the head revealed an osteolytic intradiploic lesion of the cranial vault. The lesion was excised in toto, and histopathological examination revealed benign intradiploic meningioma. The possible etiology, clinical findings, CT appearance, differential diagnosis, and treatment strategy are discussed.

In cases of pineal tumor combined with obstructive hydrocephalus, preoperative ventriculostomy or ventriculoperitoneal shunting is typically required prior to tumor resection. The objectives of preoperative ventriculostomy are gradual reduction of intracranial pressure and consequent preoperative brain protection. Here we report a case of pineal tumor resection with preoperative ventriculostomy that was complicated by multiple epidural hematomas. While postoperative intracranial hemorrhage may occur at any site, it is rare in those areas remote from the operative field. In the present case, multiple remote sequential epidural hematomas developed following resection of a pineal gland tumor. We also discuss the pathophysiologic mechanisms and provide a literature review.

Surgery for skull base lesions has advanced considerably in the past few years. The improvement in surgical results could be attributed to the availability of refined imaging modalities, modern technological advances and multidisciplinary team approach. In this report, we present our personal experience in the surgical management of 45 children with a variety of skull base lesions treated over 10 years. This article includes a retrospective analysis of the surgical approaches used and their results with a review of the literature.

Post-traumatic rhinnorhea due to large frontobasal fractures remains a difficult entity to treat. The authors report the case of a 9-year-old boy who had persistent CSF rhinnorhea due to extensive frontobasal fractures and who was managed with transcranial extradural surgery with titanium mesh placement and only pedicled pericranial flap.